| dbo:abstract |
Protein-S-Mangel ist eine angeborene oder erworbene Bluterkrankung mit einem deutlich erhöhten Risiko von Beinvenenthrombosen. (de) Protein S deficiency is a disorder associated with increased risk of venous thrombosis. Protein S, a vitamin K-dependent physiological anticoagulant, acts as a nonenzymatic cofactor to activate protein C in the degradation of factor Va and factor VIIIa. Decreased (antigen) levels or impaired function of protein S leads to decreased degradation of factor Va and factor VIIIa and an increased propensity to venous thrombosis. Protein S circulates in human plasma in two forms: approximately 60 percent is bound to complement component C4b β-chain while the remaining 40 percent is free, only free protein S has activated protein C cofactor activity (en) Le déficit en protéine S (protéine anti-coagulante) est une maladie génétique rare source de thrombophilie (risque de thromboses). Le risque thrombotique est surtout veineux (maladie thromboembolique). Le déficit peut être soit quantitatif soit qualitatif. La forme homozygote est létale ; la forme hétérozygote donne lieu à des thromboses récidivantes dans la moitié des cas. Le facteur V Leiden qui consiste en une résistance du facteur V à la protéine C (dont la protéine S est un cofacteur) donne des symptômes similaires mais moins prononcés. La protéine S étant vitamine K dépendant, les anticoagulants oraux diminuent son taux ; c'est ce qui explique l'effet procoagulant initial de ces médicaments. (fr) Il deficit di proteina S è un disturbo associato all'aumento di rischio di incorrere in trombosi venosa. La proteina S, un anticoagulante fisiologico dipendente dalla vitamina K, agisce come cofattore enzimatico di proteina C attivata nella degradazione proteolitica del fattore V e del Fattore VIII. La diminuzione dei livelli o l'alterata funzionalità della proteina S comporta alla diminuzione della degradazione del fattore Va e del fattore VIII e quindi ad un aumento della tendenza alla trombosi venosa. La proteina S circola nel plasma umano in due forme. (it) |
| dbo:diseasesDB |
10814 |
| dbo:eMedicineSubject |
med (en) |
| dbo:eMedicineTopic |
1924 (en) |
| dbo:icd10 |
D68.5 |
| dbo:icd9 |
289.81 |
| dbo:medicalCause |
dbr:Vitamin_K_deficiency |
| dbo:meshId |
D018455 |
| dbo:omim |
176880 (xsd:integer) |
| dbo:symptom |
dbr:Purpura_fulminans |
| dbo:thumbnail |
wiki-commons:Special:FilePath/Protein_PROS1_PDB_1z6c.png?width=300 |
| dbo:treatment |
dbr:Warfarin dbr:Heparin |
| dbo:wikiPageExternalLink |
https://th.schattauer.de/en/contents/archive/issue/740/manuscript/8585.html%7Caccess-date=16 |
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dbr:Protein_C dbr:Purpura_fulminans dbr:Endothelium dbr:Blood_clots dbr:Antigen dbr:Homozygotes dbr:Vitamin_K dbr:Protein_S dbr:Gene dbr:Antiphospholipid_syndrome dbr:Autosomal dbc:Coagulopathies dbr:Warfarin dbr:Cytogenetic dbr:Amino_acids dbr:Dabigatran dbr:Chromosome_3 dbr:Prophylaxis dbr:Protein dbr:Heparin dbr:Coagulation dbr:Disseminated_intravascular_coagulation dbr:Dominance_(genetics) dbr:Antithrombin_deficiency dbr:Factor_Xa dbr:Factor_V dbr:Factor_VIII dbr:Liver_disease dbr:Vitamin_K_deficiency dbr:Thrombosis dbr:Superficial_thrombophlebitis dbr:Venous_thrombosis dbr:PROS1 dbr:Zn2+ dbr:Thrombotic_disease dbr:Beta_chain dbr:Antiphospholipid_antibodies dbr:Liver_cells dbr:File:Dabigatran_structure.svg dbr:File:Human_male_karyotpe_high_resolution_-_Chromosome_3_cropped.png dbr:File:Blue_Top.JPG |
| dbp:caption |
Protein S structure (en) |
| dbp:causes |
Vitamin K deficiency (en) |
| dbp:diagnosis |
Coagulation test (en) |
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10814 (xsd:integer) |
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med (en) |
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1924 (xsd:integer) |
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289.810000 (xsd:double) (en) D68.5 (en) |
| dbp:meshid |
D018455 (en) |
| dbp:name |
Protein S deficiency (en) |
| dbp:omim |
176880 (xsd:integer) |
| dbp:snomedCt |
1563006 (xsd:integer) |
| dbp:symptoms |
Purpura fulminans (en) |
| dbp:treatment |
Heparin, Warfarin (en) |
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| dbp:wordnet_type |
http://www.w3.org/2006/03/wn/wn20/instances/synset-disease-noun-1 |
| dct:subject |
dbc:Coagulopathies |
| gold:hypernym |
dbr:Disorder |
| rdf:type |
owl:Thing wikidata:Q12136 yago:WikicatBloodDisorders yago:Abstraction100002137 yago:Attribute100024264 yago:BloodDisease114189204 yago:BloodDyscrasia114053850 yago:Condition113920835 yago:Disease114070360 yago:Dyscrasia114053717 yago:IllHealth114052046 yago:Illness114061805 yago:PathologicalState114051917 yago:PhysicalCondition114034177 dbo:Disease yago:State100024720 |
| rdfs:comment |
Protein-S-Mangel ist eine angeborene oder erworbene Bluterkrankung mit einem deutlich erhöhten Risiko von Beinvenenthrombosen. (de) Il deficit di proteina S è un disturbo associato all'aumento di rischio di incorrere in trombosi venosa. La proteina S, un anticoagulante fisiologico dipendente dalla vitamina K, agisce come cofattore enzimatico di proteina C attivata nella degradazione proteolitica del fattore V e del Fattore VIII. La diminuzione dei livelli o l'alterata funzionalità della proteina S comporta alla diminuzione della degradazione del fattore Va e del fattore VIII e quindi ad un aumento della tendenza alla trombosi venosa. La proteina S circola nel plasma umano in due forme. (it) Le déficit en protéine S (protéine anti-coagulante) est une maladie génétique rare source de thrombophilie (risque de thromboses). Le risque thrombotique est surtout veineux (maladie thromboembolique). Le déficit peut être soit quantitatif soit qualitatif. La forme homozygote est létale ; la forme hétérozygote donne lieu à des thromboses récidivantes dans la moitié des cas. Le facteur V Leiden qui consiste en une résistance du facteur V à la protéine C (dont la protéine S est un cofacteur) donne des symptômes similaires mais moins prononcés. (fr) Protein S deficiency is a disorder associated with increased risk of venous thrombosis. Protein S, a vitamin K-dependent physiological anticoagulant, acts as a nonenzymatic cofactor to activate protein C in the degradation of factor Va and factor VIIIa. (en) |
| rdfs:label |
Protein-S-Mangel (de) Déficit en protéine S (fr) Deficit di proteina S (it) Protein S deficiency (en) |
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freebase:Protein S deficiency http://www4.wiwiss.fu-berlin.de/diseasome/resource/diseases/3668 http://www4.wiwiss.fu-berlin.de/diseasome/resource/diseases/961 http://purl.org/net/tcm/tcm.lifescience.ntu.edu.tw/id/disease/Protein_Deficiency wikidata:Protein S deficiency http://bs.dbpedia.org/resource/Nedostatak_proteina_S dbpedia-de:Protein S deficiency dbpedia-fr:Protein S deficiency dbpedia-it:Protein S deficiency https://global.dbpedia.org/id/2pbgo |
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wikipedia-en:Protein_S_deficiency?oldid=1068919607&ns=0 |
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wiki-commons:Special:FilePath/Blue_Top.jpg wiki-commons:Special:FilePath/Dabigatran_structure.svg wiki-commons:Special:FilePath/Human_male_karyotpe_high_resolution_-_Chromosome_3_cropped.png wiki-commons:Special:FilePath/Protein_PROS1_PDB_1z6c.png |
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wikipedia-en:Protein_S_deficiency |
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Protein S deficiency (en) |
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dbr:PSD |
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dbr:Protein_S_Deficiency dbr:Protein_s_deficiency |
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dbr:Protein_S_Deficiency dbr:Pulmonary_embolism dbr:List_of_diseases_(P) dbr:List_of_genetic_disorders dbr:Deep_vein_thrombosis dbr:List_of_hematologic_conditions dbr:Protein_S dbr:Pre-existing_disease_in_pregnancy dbr:Prothrombin_fragment_1+2 dbr:Fresh_frozen_plasma dbr:Protein_s_deficiency dbr:Calciphylaxis dbr:Hematologic_disease dbr:PSD dbr:Activated_protein_C_resistance dbr:Thrombotic_storm dbr:Feminizing_hormone_therapy dbr:Chromosome_3 dbr:Coagulation dbr:Polyestradiol_phosphate dbr:Konrad_Plewa dbr:Sticky_platelet_syndrome dbr:Thrombophlebitis dbr:Thrombophilia dbr:Venous_thrombosis dbr:Warfarin_necrosis |
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